The wolf's bite disease

December 16, 2024 - 08:06
SLE, or lupus, is a chronic autoimmune disease in which the immune system mistakenly attacks healthy tissues, leading to widespread inflammation.

 

Dr Mattias Larsson. Photo courtesy of Family Medical Practice

Dr Mattias Larsson*

Lan, a vibrant and active 14-year-old girl, had been struggling with slow growth and poor weight gain for several years. Her parents assumed these issues would improve with time, as she was generally healthy and ate well.

Over recent months, Lan began to experience troubling symptoms: constant fatigue, a feeling of being unusually cold, generalised joint pain and a lack of appetite. Her school performance began to decline and she grew increasingly self-conscious about the erratic rashes appearing on her face.

Concerned, her parents sought help from a local doctor, who diagnosed Lan with an infection and prescribed antibiotics, steroids and vitamins. While the treatment brought temporary relief, her symptoms returned and worsened once the medication course ended. Desperate for answers, her family decided to consult the Family Medical Practice (FMP), known for its expertise in paediatric care.

At FMP, the paediatrician reviewed her history and performed a thorough physical examination. The findings were concerning: Lan showed signs of delayed puberty, with sparse axillary and pubic hair and minimal breast development. Her dry skin, brittle hair, and cold extremities hinted at potential endocrine issues, while bluish skin on hands, feet, or limbs due to poor circulation or lack of oxygen peripheral cyanosis raised concerns about malnutrition. Additionally, the bloating or swelling of her abdomen abdominal distension and facial rash suggested malabsorption syndromes, such as celiac disease.

The paediatrician considered several possible causes for Lan’s condition. These included thyroid hormone deficiency, blood cancer, health issues that occur after infections with the CMV or EBV virus, post-infectious syndromes after CMV or EBV virus infections, autoimmune disorders, anorexia nervosa and inflammatory bowel disease.

To narrow down the possibilities, the doctor ordered a series of tests. The results revealed anemia with low haemoglobin, mild swelling inflammation with higher elevated levels of a blood marker (CRP), mild inflammation with elevated CRP, and protein in the urine, indicating potential kidney involvement. While her thyroid hormones were normal, her high erythrocyte (red blood cell) test results, sedimentation rate  (ESR) and low platelet count suggested ongoing inflammation; her elevated ESR and low platelet count pointed to chronic inflammation.

A positive antinuclear antibody (ANA) test further suggested an autoimmune condition. Additional testing for anti-dsDNA antibodies confirmed the diagnosis: systemic lupus erythematosus (SLE).

The paediatrician explained the diagnosis to Lan and her family. SLE, or lupus, is a chronic autoimmune disease in which the immune system mistakenly attacks healthy tissues, leading to widespread inflammation. Its symptoms vary widely but often include fatigue, joint pain, fevers, hair loss, mouth ulcers, swollen lymph nodes and the characteristic butterfly-shaped rash on the face. SLE typically occurs in episodes of flare-ups followed by periods of remission.

Although the exact cause of SLE remains unclear, genetic and environmental factors are thought to play a role. Among identical twins, if one develops SLE, there is a 24 per cent chance the other will, and the disease is more common in women and people of African, Caribbean, or Chinese descent. Diagnosing SLE is complex, relying on a combination of clinical observations and laboratory findings. Lan’s case met six of the eleven diagnostic criteria for SLE, including the butterfly rash, kidney disease, blood disorders, arthritis and a positive ANA test.

To manage her condition, Lan was started on a treatment plan that included: Chloroquine for long-term disease control. NSAIDs to alleviate inflammation. Corticosteroids for acute symptom relief. Methotrexate, an immunosuppressant, to reduce immune system overactivity. The doctor also advised caution, as immunosuppressive therapy increases the risk of infections.

When Lan’s parents inquired about her prognosis, the pediatrician reassured them. With modern treatment options, 80–90 per cent of individuals with SLE can expect to live a normal lifespan. Lan, curious about the name "lupus," learned that it comes from the Latin word for "wolf," inspired by the facial rash’s resemblance to a wolf’s bite as perceived in medieval times. Family Medical Practice

*Dr Mattias Larsson is a paediatric doctor at Family Medical Practice and associate professor at Karolinska Institute and has a long experience in research on infectious diseases. He has worked with the Oxford University Clinical Research Unit and the Ministry of Health of Việt Nam. He is fluent in English, Swedish, Vietnamese, German and speaks some Spanish.

Family Medical Practice - FMP Healthcare Group operates medical centres in major cities including HCM City, Hà Nội and Đà Nẵng, offering consultations with international doctors, check-up centres and emergency ambulance services.

In HCM City, we have clinics in Districts 1, 2, and 7, along with the Care 1 - Executive Health Check-Up Centre and internationally accredited *9999 emergency ambulance services.

 

 

 

 

 

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